| |
n.
|
%
|
|---|
|
Classification
| | |
|
Primary PAP
| | |
|
Idiopathic + autoimmune1
|
73
|
90
|
|
Hereditary2
|
1
|
1
|
|
Secondary PAP3
|
3
|
4
|
|
PAP-like4
|
4
|
5
|
|
Gender
| | |
|
Male
|
54
|
66.5
|
|
Female
|
27
|
33.5
|
|
Smoking status
| | |
|
Current
|
18
|
22
|
|
Former
|
34
|
42
|
|
Never
|
29
|
36
|
- 1 We began to measure serum GMAb level in 2004, thus for accuracy in this group we included patients with idiopathic PAP (n = 42, those diagnosed before 2004) and patients with autoimmune PAP (n = 31, those diagnosed after 2004), although currently they are considered synonymous. 2 This subject was affected by PAP due to a GM-CSF receptor αchain mutation[17]. 3 This group includes two subjects with PAP secondary to hematologic disorders (1 chronic myeloid leukemia and 1 myelodysplastic syndrome) and 1 subject affected by lysinuric protein intolerance (the latter’s case history has already been reported)[18, 19]. 4 This group includes four subjects with a mutation in the ABCA3 gene (manuscript in preparation).
